Pediatric Cardiology and Cardiac Surgery
Vol.25 No.1 2009 (8-15)

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Sudden cardiac death (SCD) is an uncommon but extremely tragic event in pediatric patients. In this review, we discuss recent advances in this problem, focusing on the cardiovascular diseases associated with SCD and the management of patients who are identified as being at high risk for SCD. The most significant advances have occurred in the following areas: 1) definition of the causes and treatments of genetic cardiovascular diseases; 2) advances in risk assessment for SCD in patients with congenital heart disease (CHD); and 3) treatment strategies for SCD risk reduction in patients with cardiomyopathies. Based on recent advances, our understanding of the causes and risk factors for SCD as well as management of children at risk has improved. This includes clinical criteria for SCD risk stratification as well as optimal management strategies such as the judicious use of anti-arrhythmic medications and implantable device therapy.